Cogan’s syndrome (CS) is an autoimmune condition. Characteristic features of CS include inflammation of the inner ears and eyes, which can lead to vision difficulties, hearing loss, and dizziness.

Cogan’s syndrome is an inflammatory condition that involves damage to the inner ears and eyes. Widespread inflammation may also affect other organs, such as blood vessels.

Symptoms of the condition often involve eye pain and irritation, vision problems, hearing loss, and vertigo. The exact cause of Cogan’s syndrome is not well understood, but it likely occurs due to an autoimmune response, where the immune system mistakenly attacks the tissues of the eyes and ears.

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Cogan’s syndrome is a rare rheumatic condition that affects the eyes and inner ears. A rheumatic condition describes a disorder where widespread inflammation, in areas of the body where it is not necessary, causes damage and resulting symptoms.

In addition to affecting the eyes and inner ears, Cogan’s syndrome may also cause vasculitis — inflammation of blood vessels. This typically affects the larger vessels, such as the aorta in the heart, which carries oxygenated blood to the rest of the body.

Originally, Dr. Morgan and Dr. Baumgartner described the condition in 1934 as nonsyphilitic interstitial keratitis. However, Dr. David Cogan was later able to define it as a clinical entity in 1945.

At present, researchers do not understand the exact cause of Cogan’s syndrome. Evidence suggests that it occurs due to an autoimmune response that causes the immune system to mistakenly attack the tissues of the eyes and ears.

Cogan’s syndrome is not known to run in families and may develop after an infection. The condition can affect anyone but appears to be more prevalent in young adults, particularly in their 20s and 30s. Some evidence also suggests it is more common in the white population.

Common symptoms of Cogan’s syndrome may include:

  • blurry vision or red, painful, light-sensitive eyes
  • hearing loss, which may become permanent
  • vertigo, also known as dizziness
  • balance problems
  • nausea and vomiting
  • fever
  • fatigue
  • weight loss

Typically, the condition will start with either eye or inner ear inflammation. However, it often progresses to involve both. A person may also experience vasculitis symptoms at the onset of Cogan’s syndrome, or it may develop later. In some people, their symptoms may come and go.

Healthcare professionals may refer to the characteristic eye and ear symptoms as interstitial keratitis and vestibular dysfunction. Interstitial keratitis describes nonulcerative inflammation of the mid-stroma — middle layers of the cornea in the eye. Vestibular dysfunction refers to problems with the body’s balance system.

Currently, there is no single test to diagnose Cogan’s syndrome. To help diagnose Cogan’s syndrome, a doctor will consider a person’s medical history, perform a physical exam, and request tests, such as blood tests, which look for a specific antibody related to Cogan’s syndrome.

Typically, a combination of symptoms associated with the eye and inner ear may indicate Cogan’s syndrome. However, until the condition affects both the eye and inner ear, it may be difficult to reach a diagnosis. As such, a doctor may also rule out other conditions with similar symptoms. These may include:

A doctor can also confirm a diagnosis of vasculitis in individuals with Cogan’s syndrome after an exam by an ophthalmologist and otolaryngologist, as well as by imaging studies and biopsy.

Potential complications of Cogan’s syndrome typically involve visual, auditory, or balance problems. For example, it is common for a person with Cogan’s syndrome to experience permanent hearing loss. Episodes of vertigo may improve over time, but poor balance can persist. Permanent eye damage and vision loss are uncommon.

If a person with Cogan’s syndrome experiences vasculitis that affects their aorta, it can lead to congestive heart failure. Although life threatening complications are rare, they can include an aortic aneurysm. This refers to an unusual bulge in a weakened artery wall that can rupture.

If a person experiences any significant visual or auditory problems or difficulties with balance, it is advisable to contact a doctor.

Cogan’s syndrome is often a lifelong condition. However, symptoms may come and go, and they often respond well to treatment.

For mild eye disease, a doctor may initially treat the condition with anti-inflammatory medications, such as nonsteroidal anti-inflammatory drugs (NSAIDs). If an infection is causing eye inflammation, a doctor may recommend antibiotics. In people with more severe cases, a doctor may prescribe oral corticosteroids. If a person does not respond well to steroids, a doctor may recommend other immunosuppressants.

If excess fluid in the ear is causing balance problems, a doctor may suggest diuretics. They may also recommend antihistamines, benzodiazepines, and bed rest.

If medications are not helping with hearing problems, cochlear implants may be helpful. These are small devices that translate sounds into electrical signals that transmit to the brain. A surgeon implants part of the device in the ear, and the other part sits behind the ear.

Other people may just require traditional hearing aids.

If inflammation severely damages the eye, a corneal transplant may help improve vision. This surgery involves replacing the damaged cornea with one from an organ donor.

Cogan’s syndrome is an autoimmune condition that affects the eyes and inner ear. Researchers are unsure of the exact cause of the condition, but characteristic features include vision difficulties, hearing loss, and vertigo.

Cogan’s syndrome is a chronic condition, but it often responds well to treatment. This may include a combination of medications and surgery. Potential complications of Cogan’s syndrome may include permanent hearing loss and heart problems.